Systemic sclerosis is an autoimmune inflammatory disorder of unknown etiologycharacterized b y pronounced fibroproliferative alterations in the microvasculature, and frequent cellular and humoral immunity abnormalities, culminating in a severe and often progressive fibrotic process. Numerous biomarkers reflecting the three main pathogenetic mechanisms in systemic sclerosis have been described; ...

Skin tumors occurring on the scleroderma fingers are rarely seen. Swollen fingers are hallmarks of systemic sclerosis, and mucin deposition in the lesional skin is a constant feature in systemic sclerosis. Here we describe a case of cutaneous myxoid cyst on the flexor aspect of the sclerotic fingers in a patient with severe diffuse cutaneous systemic sclerosis. Cutaneous myxoid cyst is a relati...

Pneumatosis cystoides intestinalis (PCI) is the rare clinical finding of gas accumulation within the gastrointestinal wall. It can be secondary to a variety of disease processes including Crohn’s disease and systemic sclerosis. The present case describes PCI associated with pneumoperitoneum and ascites as the initial presentation of systemic sclerosis in a 64-year-old female with a history of C...

Without doubt, animal models have provided significant insights into our understanding of the rheumatological diseases; however, no model has accurately replicated all aspects of any autoimmune disease. Recent years have seen a plethora of knockouts and transgenics that have contributed to our knowledge of the initiating events of systemic sclerosis, an autoimmune disease. In this review, the f...

Systemic sclerosis sine scleroderma is a rare form of limited systemic sclerosis. These patients are without skin involvement, but do not differ in its clinical or laboratory features and prognosis from classical systemic sclerosis. In the absence of cutaneous signs/symptoms, its diagnosis is delayed leading to significant morbidity and mortality. We report a case of sixty year old female who p...

Several occupational hazards, especially exposure to silica, have been implicated as causal factors for the development of scleroderma-like disorders. Compared to other connective tissue disorders, silica-associated systemic sclerosis (SA-SS) is relatively rare. Silica-induced scleroderma is indistinguishable from idiopathic systemic sclerosis. However, the former expresses a high predispositio...

As a measure of enterocyte function, the deconjugation of pteroyl-L-glutamyl-y-Lglutamyl-y-L-glutamic acid to folic acid and subsequent active absorption was measured in 19 patients with progressive systemic sclerosis and compared with 14 controls. The absorption step of folic acid was identical in the two groups, while deconjugation of pteroyl-L-glutamyl-y-L-glutamyly-L-glutamic acid was signi...

OBJECTIVE To determine the presence of antiubiquitin antibody (AUbA) in localised scleroderma and systemic sclerosis, as it is frequently found in the sera of patients with systemic lupus erythematosus (SLE) and has also been shown to have a close relationship with antihistone antibodies that have an important role in scleroderma. METHODS Serum samples from patients with localised scleroderma...

Systemic sclerosis is a disease which usually progresses or reaches a plateau with persistence of symptoms and signs. Regression is extremely unusual. Four cases of established scleroderma are described in which regression is well documented. The significance of this observation and possible mechanisms of disease regression are discussed.

Systemic scleroderma (SSc) is one of the most complex systemic autoimmune diseases. It targets the vasculature, connective tissue-producing cells (namely fibroblasts/myofibroblasts), and components of the innate and adaptive immune systems. Clinical and pathologic manifestations of SSc are the result of: (1) innate/adaptive immune system abnormalities leading to production of autoantibodies and...